通讯作者: 黎承平▲

DOI：10.12201/bmr.202505.00006

Recent advances in the treatment of acquired hemophilia A.

• 摘要：获得性血友病A（Acquired Hemophilia A，AHA）是一种罕见的出血性疾病，其特征是血液循环中出现针对凝血因子Ⅷ（Coagulation factor Ⅷ，FⅧ）的自身抗体，导致FⅧ活性降低。该病各年龄段均可发生，多见于老年人和产后妇女，临床表现为自发性出血或外伤后严重出血。治疗AHA的核心在于迅速而准确的诊断，并及时采取治疗措施。治疗方法包括控制急性出血、清除抑制物和积极治疗原发病。控制出血的措施包括替代治疗和旁路途径药物治疗。清除抑制物主要依靠免疫抑制治疗（immunosuppressive therapy，IST），包括一线和二线治疗方案。近年来，新的治疗方法也显示出良好的疗效和安全性，为AHA患者提供了新的治疗选择。

  关键词： 获得性血友病A; 凝血因子Ⅷ; 治疗; 出血性疾病

   

  Abstract: Acquired Hemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies against coagulation FⅧ in the bloodstream, leading to decreased coagulation FⅧ activity. AHA occurs in all age groups, but it is most commonly seen in the elderly and postpartum women. Clinically, it is characterized by spontaneous bleeding or severe bleeding following trauma. The treatment for AHA focuses on obtaining a rapid and accurate diagnosis and implementing timely therapeutic measures. Treatment involves managing acute bleeding, removing inhibitors, and aggressively addressing the primary disease. To control bleeding, alternative therapies such as recombinant porcine FⅧ, desmopressin (DDAVP), and emicizumab can be used, along with bypass pathway drugs. The removal of inhibitors mainly depends on immunosuppressive therapies, including first- and second-line treatment options. In recent years, newer therapies such as CD38 monoclonal antibodies and the CyDRi regimen (cyclophosphamide, dexamethasone, and rituximab) have demonstrated promising efficacy and safety profiles. These treatments offer new options for patients with AHA.

  Key words: Acquired hemophilia A; Coagulation factor Ⅷ; Treatment；Hemorrhagic disorder; 

  提交时间：2025-05-10

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• 序号	提交日期	编号	操作
  1	2025-03-09	10.12201/bmr.202505.00006V1	下载

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